ArticleArthritis & rheumatology (Hoboken, N.J.)2026
Interstitial Lung Disease in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A European Multicenter Study.
Article in Arthritis & rheumatology (Hoboken, N.J.), 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
2 citing papers in PubMed.
- Reply.Arthritis & rheumatology (Hoboken, N.J.) · 2026Article
- Interstitial lung disease in ANCA-associated vasculitis: comment on the article by Chalkia et al.Arthritis & rheumatology (Hoboken, N.J.) · 2026Article
Corrections and comments
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Authors and funding
19 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveInterstitial lung disease (ILD) can occur in association with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV-ILD) or as an isolated entity with positive ANCA (ANCA-ILD). However, data on the epidemiology and outcomes of these conditions remain limited.
methodsA European multicenter retrospective study encompassed patients with AAV-ILD or ANCA-ILD. Baseline and subsequent chest computed tomography studies were centrally reviewed. Primary outcomes included forced vital capacity % (FVC%) decline, respiratory failure, and mortality.
resultsOf 162 patients (myeloperoxidase [MPO]-ANCA 85%), 123 (76%) had AAV-ILD and 39 (24%) ANCA-ILD. At baseline, usual interstitial pneumonia (UIP) was the most frequent radiologic pattern (57%), whereas half had a radiologic fibrosis grade >10%. Kidney involvement was present in 73%, most commonly Berden focal class. UIP and nonspecific interstitial pneumonia (NSIP) patterns showed greater annual FVC% decline than other patterns (UIP -1.99%, NSIP -3.76% [P = 0.35], others +0.36%). An adjusted mixed-effects model indicated that rituximab was associated with mean FVC% improvement at 12 months (+6.02%; P = 0.07). Radiologic progression occurred in ~50%, mainly in younger patients with a higher fibrosis severity grade. Respiratory failure (19%) was associated with fibrosis severity (grade 4 hazard ratio [HR] 4.7, P = 0.029) and baseline FVC% (HR 0.95, P = 0.002). Over a median 4.2-year follow-up, 48% died. Age (HR 1.08, P = 0.04) and baseline FVC% (HR 0.97, P = 0.05) were independent predictors of mortality.
conclusionAt baseline, higher fibrosis severity, UIP, and lower FVC% were associated with worse outcomes. Immunosuppressives, such as rituximab, may help preserve lung function. The need for early identification and individualized treatment in ILD associated with AAV or ANCA is underscored.
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